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Review Article Patient and Tumour Characteristics of Adult Head and Neck Soft Tissue Sarcomas: A Systematic Review and Meta-Analysis Sakshi Andersen, 1 Henriette Mann, 1 Anders Krarup-Hansen , 2 Christel Bræmer Lajer, 1 and Christian Grønhøj 1 1 Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, University of Copenhagen, Blegdamsvej 9, 2100 Copenhagen, Denmark 2 Department of Oncology, Herlev Hospital, Herlev Ringvej 75, 2730 Herlev, Denmark Correspondence should be addressed to Christian Grønhøj; [email protected] Received 10 January 2019; Revised 17 March 2019; Accepted 31 March 2019; Published 22 May 2019 Academic Editor: C. Verhoef Copyright © 2019 Sakshi Andersen et al. is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Background. Head and neck soft tissue sarcomas (HNSTS) constitute a rare and heterogeneous cancer entity. Management remains a challenge due to the rarity and varied biological behaviour among various subtypes. is systematic review examines the characteristics of tumours and patients with HNSTS. Materials and Methods. A systematic literature review and meta-analysis were performed using the electronic databases PubMed and Embase. Eight eligible studies were identified, and 13 variables were extracted from each study including 5-year overall survival (OS) rate and 5-year disease-free survival (DFS) rate. Results. We identified eight studies (n 1,120 patients; 739 males (66%)) from six different countries). In total, 24 histological subtypes were found, and 20% of the sarcomas (n 215) could not be subclassified. 607 sarcomas (57%) were <5 cm in diameter, and 945 sarcomas (84%) were grade 3. 1,059 patients (90%) underwent surgery. Estimated 5-year OS was 74% (95% CI; 0.63–0.84%) and 5- year DFS was 56% (95% CI; 38–74%). Conclusion. HNSTS holds a relative poor prognosis possibly explained by the heterogeneity of the disease. Treatment of HNSTS has shown to be highly diverse, underlining the importance of uniformed treatment guidelines in order to achieve improved survival outcomes. 1. Introduction Head and neck soft tissue sarcomas (HNSTS) are a rare and heterogeneous group of malignancies accounting for approximately 1% of all head and neck malignancies. HNSTS represents 10% of all soft tissue sarcomas [1]. More than 80 histological subtypes are distinguished in the 2013 WHO classification [2]. Because of this rarity and the diverse clinical behaviours, management of HNSTS can be challenging and should be carried out in a mul- tidisciplinary centre with expertise and experience in sarcomas [3]. Surgery remains to be the primary treatment choice, even though it is difficult to achieve free surgical margins due to the anatomy of the head and neck region [4]. When combined with surgery, radiotherapy may improve overall prognosis for some HNSTS depending on the histological type, whereas a better outcome is achieved for some his- tological subtypes when surgery is combined with chemo- therapy, e.g., angiosarcoma, rhabdomyosarcoma, and synovial sarcoma [3, 5, 6]. e purpose of this study was to systematically review the literature on adult patients diagnosed with HNSTS and report the distribution of histological subtypes, treatment, and overall survival (OS) rates. 2. Materials and Methods 2.1. Search Strategy and Eligibility Criteria. e electronic databases PubMed and Embase were used for searching. e search strategy in PubMed included the following keywords: “Head and Neck,” “Oral Cavity,” “Pharynx,” and Hindawi Sarcoma Volume 2019, Article ID 9725637, 8 pages https://doi.org/10.1155/2019/9725637

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Page 1: ReviewArticle - Hindawi Publishing Corporationdownloads.hindawi.com/journals/sarcoma/2019/9725637.pdf · ReviewArticle PatientandTumourCharacteristicsofAdultHeadandNeckSoft TissueSarcomas:ASystematicReviewandMeta-Analysis

Review ArticlePatient and Tumour Characteristics of Adult Head and Neck SoftTissue Sarcomas: A Systematic Review and Meta-Analysis

Sakshi Andersen,1 Henriette Mann,1 Anders Krarup-Hansen ,2 Christel Bræmer Lajer,1

and Christian Grønhøj 1

1Department of Otorhinolaryngology, Head and Neck Surgery and Audiology, Rigshospitalet, University of Copenhagen,Blegdamsvej 9, 2100 Copenhagen, Denmark2Department of Oncology, Herlev Hospital, Herlev Ringvej 75, 2730 Herlev, Denmark

Correspondence should be addressed to Christian Grønhøj; [email protected]

Received 10 January 2019; Revised 17 March 2019; Accepted 31 March 2019; Published 22 May 2019

Academic Editor: C. Verhoef

Copyright © 2019 Sakshi Andersen et al. ,is is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work isproperly cited.

Background. Head and neck soft tissue sarcomas (HNSTS) constitute a rare and heterogeneous cancer entity. Managementremains a challenge due to the rarity and varied biological behaviour among various subtypes.,is systematic review examines thecharacteristics of tumours and patients with HNSTS. Materials and Methods. A systematic literature review and meta-analysiswere performed using the electronic databases PubMed and Embase. Eight eligible studies were identified, and 13 variables wereextracted from each study including 5-year overall survival (OS) rate and 5-year disease-free survival (DFS) rate. Results. Weidentified eight studies (n� 1,120 patients; 739 males (66%)) from six different countries). In total, 24 histological subtypes werefound, and 20% of the sarcomas (n� 215) could not be subclassified. 607 sarcomas (57%) were <5 cm in diameter, and 945sarcomas (84%) were grade 3. 1,059 patients (90%) underwent surgery. Estimated 5-year OS was 74% (95% CI; 0.63–0.84%) and 5-year DFS was 56% (95% CI; 38–74%). Conclusion. HNSTS holds a relative poor prognosis possibly explained by the heterogeneityof the disease. Treatment of HNSTS has shown to be highly diverse, underlining the importance of uniformed treatment guidelinesin order to achieve improved survival outcomes.

1. Introduction

Head and neck soft tissue sarcomas (HNSTS) are a rareand heterogeneous group of malignancies accounting forapproximately 1% of all head and neck malignancies.HNSTS represents 10% of all soft tissue sarcomas [1].More than 80 histological subtypes are distinguished inthe 2013WHO classification [2]. Because of this rarity andthe diverse clinical behaviours, management of HNSTScan be challenging and should be carried out in a mul-tidisciplinary centre with expertise and experience insarcomas [3].

Surgery remains to be the primary treatment choice,even though it is difficult to achieve free surgical margins dueto the anatomy of the head and neck region [4]. Whencombined with surgery, radiotherapy may improve overall

prognosis for some HNSTS depending on the histologicaltype, whereas a better outcome is achieved for some his-tological subtypes when surgery is combined with chemo-therapy, e.g., angiosarcoma, rhabdomyosarcoma, andsynovial sarcoma [3, 5, 6].

,e purpose of this study was to systematically reviewthe literature on adult patients diagnosed with HNSTS andreport the distribution of histological subtypes, treatment,and overall survival (OS) rates.

2. Materials and Methods

2.1. Search Strategy and Eligibility Criteria. ,e electronicdatabases PubMed and Embase were used for searching.,e search strategy in PubMed included the followingkeywords: “Head and Neck,” “Oral Cavity,” “Pharynx,” and

HindawiSarcomaVolume 2019, Article ID 9725637, 8 pageshttps://doi.org/10.1155/2019/9725637

Page 2: ReviewArticle - Hindawi Publishing Corporationdownloads.hindawi.com/journals/sarcoma/2019/9725637.pdf · ReviewArticle PatientandTumourCharacteristicsofAdultHeadandNeckSoft TissueSarcomas:ASystematicReviewandMeta-Analysis

Tabl

e1:

Patient

data

andcharacteristics.

Mahmou

det

al.

[7]

And

raet

al.[8]

Gon

zalez-

Gon

zalezet

al.

[9]

Vitzthum

etal.

[10]

Tejani

etal.[6]

Penele

tal.[11]

Sidapp

aand

Krishnamurthy

[12]

Park

etal.[13]

Total

Cou

ntry

USA

Germany

Mexico

USA

USA

France

India

Korea

N%

Years

2004–2

013

2003–2

012

2004–2

009

1998–2

012

1999–2

009

1997–2

002

1996–2

005

1995–2

012

N%

N%

N%

N%

N%

N%

N%

N%

Cases

788

2651

4830

2827

122

1120

Male

539

6818

6931

6134

7120

6715

5417

6365

53739

66Medianage

6364

43.1

68.5

5045.7

3546

—<5

0181

23a

—34

67—

1240

1139

——

238

27>5

0607

77—

1733

b—

1860

1761

b—

—659

73Tu

mou

rsiz

e<5

cm443

56—

1529

c33

69c

1343

—17

6386

70c

607

57>5

cm345

44d

—36

7115

3111

37—

1037

3630

453

43Meantumou

rsiz

e(cm)

4.5e

4.6e

——

—2.7

4.8

—Grade

(FNCLC

C)

High/G3

788

100

1765

3059

2858

f23

7716

5721

78g

2218

945

84Interm

ediate/G

20

831

1020

03

108

290

5343

827

Low/G

10

14

1122

613

27

27

622

3025

585

Unspecified/un

know

n0

00

1429

27

27

017

1435

3Tstage

0—

——

——

——

——

1—

1454

1325

—12

40—

——

3923

2—

1246

3875

—18

60—

——

6840

3—

——

——

——

——

4—

——

——

——

——

Surgical

margins

R0497

6310

3935

6941

8517

57—

2074

7662

696

60R1

111

146

234

8—

723

—6

22h

2016

154

13R2

180

235

1913

253

6—

——

76

208

18Unspecified/un

know

n—

519

—4

8—

——

—9

15-year

OS

—82

—83

46—

6875

—5-year

DFS

——

—63

35—

65—

—Medianfollo

w-up(m

onths)

4539

23.8

57.6

—27

5972

—a ≤50,b≥5

0,c ≤5,

d ≥5,

e median,

f grade

2+3,

g grading

system

notspecified

inthearticle,a

ndh R

1+R2

.†R0

�negativ

esurgical

margins/clear

margins,R

1�microscop

icpo

sitivesurgical

margins/m

icroscop

icinvolved

margins,a

ndR2

�macroscop

icpo

sitivemargins/m

acroscop

icinvolved

margins.

2 Sarcoma

Page 3: ReviewArticle - Hindawi Publishing Corporationdownloads.hindawi.com/journals/sarcoma/2019/9725637.pdf · ReviewArticle PatientandTumourCharacteristicsofAdultHeadandNeckSoft TissueSarcomas:ASystematicReviewandMeta-Analysis

“Sarcoma.” �e search strategy in Embase included thekeywords “Head and Neck Sarcomas.” Studies publishedfrom 2000 until March 2018 reporting patient databases ofsoft tissue sarcomas in the head and neck region with aminimum of 20 patients (>18 years of age at diagnosis)were included. Studies reporting both localized disease andmetastatic disease were included. Exclusion criteria wereradiation-induced sarcomas, bone sarcomas, and studiesthat solely reported speci�c histological subtypes. Due to anupdate in WHO classi�cation of soft tissue tumours in1994, studies reporting patient cases from before 1994 wereexcluded.

2.2. Data Extraction. We extracted data on country, numberof cases, period, gender, age, histological subtypes, tumoursize, and grading according to the French Federation of CancerCentres Sarcoma Group (grade I, grade II, grade III, andunknown). Furthermore, T stage (0, 1, 2, 3, and 4), treatment(surgery, radiotherapy, and/or chemotherapy), surgical mar-gins (R0, R1, R2, and unspeci�ed/unknown), median followup, 5-year OS, and 5-year disease-free survival (DFS) were alsoextracted. We applied the model of random e�ects to performa meta-analysis of 5-year OS and 5-year DFS. Data analysiswas performed in Stata, and a p value< 0.05 was consideredstatistically signi�cant.

3. Results

We identi�ed 2,056 publications, of which eight studies(1,120 cases; 66% males) from six di�erent countries met theinclusion criteria. All studies reported the median age, whichranged from 35 to 68.5 years. In four studies (n� 897), 73%of the patients were more than 50 years of age (Table 1 andFigure 1).

Sarcomas with unidenti�ed histology (n� 215) anduncommon sarcoma subtypes (n� 141) constituted one-third of the cohort (n� 1,083) being the most frequenthistological subtypes.�ese were followed by �brosarcomas/�bromatous sarcomas (n� 136), vascular sarcomas(n� 125), and leiomyosarcomas (n� 89). Noteworthy, insome of the included studies, the group “uncommon sar-comas” consisted of several sarcoma subtypes traditionallyperceived as common sarcomas (e.g., rhabdomyosarcomas)(Table 2 and Figure 2). �is was mainly seen in studiesincluding a small number of cases. Hence, several of thecases classi�ed as “uncommon sarcomas” could potentiallybe reclassi�ed within one of the speci�ed subtypes.

In seven studies (n� 1,090), the median follow-up periodranged from 23.8 to 72months. Five-year OS was reported in�ve studies, 5-year DFS was reported in three studies, andtheir values were 74% (95% CI; 0.63–0.84%) and 56 % (95%CI; 38–74%), respectively (Table 1 and Figures 3 and 4).

Records identified through PubMed and Embase (n = 2,056)

Scre

enin

gIn

clud

edEl

igib

ility

Iden

tific

atio

n Additional records identified through other sources

(n = 4)

Records after duplicates removed(n = 1,844)

Records screened on basisof title and/or abstract

(n = 1,844)

Full-text articles assessed for eligibility

(n = 14)

Studies included in qualitative synthesis

(n = 8)

Studies included in quantitative synthesis (meta-analysis)

5-year OS (n = 5)5-year DFS (n = 3)

Records excluded(n = 6)

Review (n = 3)(i)Specific histological subtype (n = 1)

(ii)

Abstract (n = 2) (iii)

Records excluded(n = 1,829)

Figure 1: PRISMA 2009 ¡ow diagram.

Sarcoma 3

Page 4: ReviewArticle - Hindawi Publishing Corporationdownloads.hindawi.com/journals/sarcoma/2019/9725637.pdf · ReviewArticle PatientandTumourCharacteristicsofAdultHeadandNeckSoft TissueSarcomas:ASystematicReviewandMeta-Analysis

Six studies (n� 1,060) reported tumour size as a binaryoutcome (<5 cm or >5 cm), two studies reported mediantumour size, and one study reported both mean tumour sizeand tumour size as a binary outcome. In 57% of the cases, thetumour diameter was <5 cm in diameter at the time ofdiagnosis. Mean tumour size ranged from 2.7 to 4.8 cm(n� 55). Tumour grading was stated in all studies included(n� 1,120). 84% of the tumours were grade III, 7% weregrade II, 5% were grade I, and 3% were unspecified orunknown grade. ,e surgical resection margins were statedin seven studies (n� 1,092). 60% of the tumours had clear

surgical margins (R0), 13% had microscopic involvedmargins (R1), 18% had macroscopic involved margins (R2),and 1% had unknown margins (Table 1).

All studies (n� 1,120) reported treatment strategy(Table 3).

4. Discussion

To our knowledge, this is the first systematic review andmeta-analysis to explore patient and tumour characteristicsincluding OS, DFS, and treatment strategies for patients with

Table 2: Histological subtypes.

Mahmoudet al. [7]

Andraet al. [8]

Gonzalez-Gonzalez et al.

[9]∗Vitzthumet al. [10]∗

Tejaniet al.[6]

Penelet al.[11]∗

Sidappa andKrishnamurthy

[12]

Parket al.[13]

Total

Alveolar soft partsarcoma — — — — 1 — — 3 4

Angiosarcoma — 9 — 14 4 5 — 15 47Carcinosarcoma — — — — 1 — — — 1Chondrosarcoma — — — — — — — 8 8DFSP▪ — — — 1 — — 16 17Epithelioid sarcoma — — — — 1 1 — — 2Ewing’s sarcoma/PNET◊ — — — — — — — 4 4

Fibrosarcoma/fibromatoussarcoma○

125 — — — 1 2 1 7 136

Histiocytic sarcoma — — — — 1 — — — 1Leiomyosarcoma 76 — — 5 3 — 1 4 89Lipomatoussarcoma∆ 31 — — 3 4 — — 10 48

Low-gradefibromyxoid sarcoma — — — — — — — 2 2

MFH/undiff. pleo./UPS∞ — 5 — 9 — 2 6 3 25

Neurofibromatoussarcoma□ 46 — 9 1 — — 5 — 61

Osteosarcoma — — — — — — — 5 5Others• — 8 — 3 — — — 5 16Peripheralneuroectodermictumour

— — — — — 3 — — 3

Rhabdomyosarcoma — — 7 1 5 7 — 24 44Spindle cell — — — 4 3 — 10 9 26Synovial sarcoma 34 4 — 6 6 2 1 7 60Uncommon sarcomasubtypes† 139 — — — — — 2 — 141

Undifferentiatedsarcoma — — — — — 3 — — 3

Unidentifiedsarcoma◆ 215 — — — — — — — 215

Vascular sarcoma‡ 122 — — 2 — — 1 — 125∗Not all of the histological subtypes were reported. ▪Dermatofibrosarcoma protuberans. ◊Primitive neuroectodermal tumour. ○Including fibrosarcomaNOS, fibromyxoma, and fibrous histiocytoma. ∆Including myxosarcoma, angiomyxoma, atypical lipoma, liposarcoma NOS, fibromyxolipoma, myxoid,round cell, pleomorphic, mixed, and dedifferentiated liposarcoma, and spindle cell lipoma. ∞MFH �malignant fibrous histiocytoma, undiff.pleo. � undifferentiated pleomorphic sarcoma. UPS � unclassified pleomorphic sarcoma. □Including neurosarcoma and malignant peripheral nervesheath tumour. •Andra et al. [8], not otherwise specified; Vitzthum et al. [10], dendritic cell sarcoma, chordoma, and hemangioengothelioma; Park et al.[13], folicular dendritic cell sarcoma and teratocarcinosarcoma. †Including clear cell, dermatofibrosarcoma, rhabdomyosarcoma, epithelioid, desmo-plastic small round cell tumour, fascial, infantile, angiomatoid, fibrous histiocytoma, rhabdoid, giant cell tumour of soft part, and alveolar soft part.◆Sarcomatosis not otherwise specified (NOS), spindle cell, giant cell, small cell, and undifferentiated sarcoma. ‡Including hemangiosarcoma, malignanthemangioendothelioma, epithelioid hemangioendothelioma, malignant hemangiopericytoma, hemangiopericytoma, and lymphangiosarcoma.

4 Sarcoma

Page 5: ReviewArticle - Hindawi Publishing Corporationdownloads.hindawi.com/journals/sarcoma/2019/9725637.pdf · ReviewArticle PatientandTumourCharacteristicsofAdultHeadandNeckSoft TissueSarcomas:ASystematicReviewandMeta-Analysis

a HNSTS. In the meta-analysis, the pooled results showed a5-year OS of approximately 75% and a 5-year DFS of nearly50%.

Surgery remains the cornerstone of treatment of HNSTS,but the close relation to vital anatomic structures compli-cates this treatment strategy, explaining the relatively low 5-

4

47

1 8 172 4

136

1

89

48

225

61

516

3

4426

60

141

3

215

125

0

50

100

150

200

Alv

eola

r sof

t par

t sar

com

a

Ang

iosa

rcom

a

Carc

inos

arco

ma

Chon

dros

arco

ma

DFS

P

Epith

elioi

d sa

rcom

a

Ewin

g's sa

rcom

a/PN

ET

Fibr

osar

com

a/fib

rom

atou

s sar

com

a

Hist

iocy

tic sa

rcom

a

Leio

myo

sarc

om

Lipo

mat

ous s

arco

ma

Low

-gra

de fi

brom

yxoi

d sa

rcom

a

MFH

/und

iff.p

leo.

/UPS

Neu

rofib

rom

atou

s sar

com

a

Oste

osar

com

a

Oth

ers

Perip

hera

l neu

roec

tode

rmic

tum

or

Rhab

dom

yosa

rcom

a

Spin

dle c

ell

Syno

vial

sarc

oma

Unc

omm

on sa

rcom

a sub

type

s

Und

iffer

entia

ted

sarc

oma

Uni

dent

ified

sarc

oma

Vasc

ular

sarc

oma

Figure 2: Histological subtypes.

Study

Andra et al. [8] 0.85 (0.65, 0.96)

ES (95% CI)

0.83 (0.70, 0.93)

0.47 (0.28, 0.66)

0.70 (0.50, 0.86)

0.75 (0.67, 0.83)

0.74 (0.63, 0.84)

Vitzthum et al. [10]

Tejani et al. [6]

Sidappa and Krishnamurthy [12]

Park et al. [13]

Overall (I2 = 71.70%, p = 0.01)

0.25 0.5 0.75Proportion

1

Figure 3: 5-year overall survival.

Study

Vitzthum et al. [10]

Tejani et al. [6]

Sidappa and Krishnamurthy [12]

Overall (I2 = 73.96%, p = 0.02)

ES (95% CI)

0.65 (0.49, 0.78)

0.37 (0.20, 0.56)

0.67 (0.46, 0.83)

0.56 (0.38, 0.74)

0.5 0.75 10.25Proportion

Figure 4: 5-year disease-free survival.

Sarcoma 5

Page 6: ReviewArticle - Hindawi Publishing Corporationdownloads.hindawi.com/journals/sarcoma/2019/9725637.pdf · ReviewArticle PatientandTumourCharacteristicsofAdultHeadandNeckSoft TissueSarcomas:ASystematicReviewandMeta-Analysis

Tabl

e3:

Treatm

ent.

Mahmou

det

al.[7]

And

raet

al.[8]

Gon

zalez-

Gon

zalez

etal.[9]

Vitzthum

etal.[10]

Tejani

etal.[6]

Penel

etal.[11]

Sidapp

aand

Krishnamurthy

[12]

Park

etal.[13]

Total

N%

N%

N%

N%

N%

N%

N%

N%

N%

Surgery

788

100

2181

4894

3063

2480

1968

2696

103

841059

90Ra

diationtherapy

414

5326

2633

6534

7118

6014

5021

7865

53625

53Chemotherapy

126

1613

1312

242

410

338

290

—42

34213

18

6 Sarcoma

Page 7: ReviewArticle - Hindawi Publishing Corporationdownloads.hindawi.com/journals/sarcoma/2019/9725637.pdf · ReviewArticle PatientandTumourCharacteristicsofAdultHeadandNeckSoft TissueSarcomas:ASystematicReviewandMeta-Analysis

year DFS. Several factors influence the degree of surgicalresection, e.g., tumour location and size, the extend of in-vasion, and the performance status of the patient [14]. Eventhough surgery with wide resection margins remains adifficult task, this review shows that 90% of the patients weretreated with surgery (± radiation therapy and/or chemo-therapy), and wide surgical margins were obtained in 60% ofthe cases.

In the present study, 53% of the patients were treatedwith radiation therapy with or without surgery. Studies inpatients with truncal or extremity soft tissue sarcomas havedemonstrated an improved local control following adjuvantradiation therapy in patients with large grade II and grade IIIsarcomas. Furthermore, retrospective studies have shown anapproximately 10% improvement in OS following adjuvantradiation therapy in patients with grade III sarcomas [15].Similarly, radiation therapy has also been found to improveOS in HNSTS [7].

In order to improve 5-year DFS and 5-year OS, the valueof neoadjuvant chemotherapy in high-graded truncal orextremity soft tissue sarcomas has been investigated.However, the study was closed prematurely because supe-riority for neoadjuvant treatment was not to be expected[16]. Nevertheless, neoadjuvant chemotherapy still holds apotential improvement in the prognosis of HNSTS, andprospective, multicentre studies are needed in order to gaininformation about the effect of neoadjuvant treatment in-cluding chemotherapy and radiation therapy. Furthermore,in a study by Blay et al., early evaluation by a multidisci-plinary team demonstrated a significant improvement inrelapse-free survival [17]. Hence, HNSTS should only bemanaged by a specialist multidisciplinary environment inorder to ensure a uniform treatment protocol [18].

More than 80 histological subtypes of HNSTS have beenidentified [2]. In order to predict risk of metastasis andrelapse, it is important to identify the exact histologicalclassification. In this systematic review, 215 sarcomas couldnot be classified, and additional 141 sarcomas were classifiedas “uncommon sarcomas.” It is remarkable that one-third ofthe cohort constituted of sarcomas with unknown histologyand uncommon sarcoma subtypes. Noteworthy, the clas-sification of soft tissue sarcomas has evolved considerablyduring the past decades, especially due to evolvement inimmunohistochemical and genetic/molecular methods [19].It is likely that a large number of the cases included in thepresent review would be reclassified if classified today.Furthermore, many of the cases where a specific pathologicalclassification could not be obtained could probably beclassified using modern methods. ,is may complicateapplying the results of the present review on newly di-agnosed patients with HNSTS. A high expertise in histo-logical classification is crucial if more knowledge aboutbehaviour and treatment of sarcomas must be achieved. Itemphasizes the need of histological reevaluation of a specialtrained pathologist in the highest national level beforetreating and before publishing data of sarcomas.

,e present systematic review and meta-analysis holdimportant limitations. Inclusion and exclusion criteria aswell as staging and treatment varied between the included

studies, which complicates interpretation. ,e includedstudies reported a few cases of sarcomas, which were notclassified as soft tissue sarcomas; however, these constitutedonly a small part (1.3%) of the total cases included. ,eincluded studies originated from six countries, and themajority of the cases were obtained from a single study. Allstudies were retrospective, and the cases were obtainedduring long follow-up periods.

5. Conclusions

HNSTS is a rare and heterogeneous tumour group with greatdifferences in OS and DFS. ,e pooled results showed a 5-year OS of approximately 75% and a 5-year DFS of nearly50%. However, studies show varying prognoses demon-strating the difficulty in treating HNSTS underlining theimportance of uniformed treatment guidelines in order toachieve improved survival outcomes.

Conflicts of Interest

,e authors declare that there are no conflicts of interestregarding the publication of this article.

Authors’ Contributions

Sakshi Andersen and Henriette Mann contributed equally tothis work.

Acknowledgments

CG was funded by the nonprofit organization CandysFoundation and Kræftfonden (the Cancer Foundation).

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[3] P. G. Casali, N. Abecassis, S. Bauer et al., “Soft tissue andvisceral sarcomas: ESMO-EURACAN clinical practiceguidelines for diagnosis, treatment and follow-up,” Annals ofOncology, vol. 29, no. 4, pp. iv51–iv67, 2018.

[4] R. H.Woods, J. A. Potter, J. L. Reid et al., “Patterns of head andneck sarcoma in Australia,” ANZ Journal of Surgery, vol. 88,no. 9, pp. 901–906, 2017.

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Sarcoma 7

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8 Sarcoma

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